Surgery or Not? A Systematic Review of Facial Congenital Melanocytic Nevi Treatment Patterns and Outcomes.

BACKGROUND: Treatment management for congenital melanocytic nevi (CMN) on the face (FCMN) is highly variable and requires a thorough assessment of multiple factors. To date, a systematic review of FCMN treatment is lacking. The purpose of the present study was to elucidate the frequency, variety, and outcomes of treatment modalities for FCMN with different levels of complexity. METHODS: A comprehensive review of Pubmed, Embase, and Google Scholar databases from 1950 to 2022 was conducted using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). Articles reporting on FCMN treatment approaches, outcomes, and associated complications were screened and data were extracted according to inclusion criteria. Data were tabulated for thematic analysis of FCMN treatment types, anatomic locations, outcomes, and complications. RESULTS: Of the 561 studies retrieved, 34 met inclusion criteria including 19 surgical treatments, 14 nonsurgical treatments, and one combined surgical and nonsurgical treatment study, totaling 356 patients. The majority of treated FCMN were small-to-medium-sized (56%). Facial CMN treated conservatively were mostly located on the cheek (27%) and/or perinasal region (21%), whereas FCMN treated with surgery were primarily located in the periorbital region (44%) and/or the cheek (17%). Across all treatment cohorts, 22% of patients experienced at least one complication, with 12% of complications experienced by patients treated by surgery. CONCLUSIONS: There is a greater need for standardized FCMN nomenclature that encompasses nevi pattern, dimensions, anatomical coverage, and quantitative measurements of treatment outcome. Future studies should focus on identifying anatomic locations of FCMN that are more prone to complications and determine which treatment approach optimizes outcomes.

Sirolimus for diffuse intestinal infantile hemangioma with PHACE features: systematic review.

BACKGROUND: We report a 3-month-old female with cardiovascular anomalies and diffuse intestinal infantile hemangioma (IIH) of the small bowel suggesting possible diagnosis of PHACE syndrome (posterior fossa anomalies, hemangioma, arterial lesions, cardiac abnormalities/coarctation of the aorta, eye anomalies). The GI symptoms persisted under treatment with propranolol, whereas the addition of sirolimus led to regression of the IIH. METHODS: A systematic review was conducted using PubMed, EMBASE, and Ovid MEDLINE databases between 1982 and 2021. RESULTS: A total of 4933 articles were identified; 24 articles met inclusion criteria with 46 IIH cases. The most common GI presentations were unspecified GI bleed (40%) and anemia (38%). The most common treatments were corticosteroids (63%), surgical resection (32.6%), and propranolol (28%). Available outcomes were primarily bleeding arrest (84%). Nine cases (19.5%) were diagnosed with definite PHACE, 5 (11%) with possible PHACE, and 32 (69.5%) no PHACE. Our case presented with symptoms most consistent with those of possible PHACE and definite PHACE. No cases in this review underwent treatment with sirolimus. CONCLUSIONS: This is the first reported case of successful treatment of IIH with sirolimus. Our case, along with other patients who present with IIH and PHACE features, suggests consideration of IIH as a diagnostic criterion for PHACE syndrome. IMPACT: This is the first reported case in which sirolimus showed regression of an intestinal infantile hemangioma. This study serves to demonstrate the presentation, treatment, outcomes of intestinal infantile hemangioma, and correlation with PHACE. The potential correlation between intestinal infantile hemangioma and PHACE deserves more study in consideration of intestinal infantile hemangioma as a diagnostic criterion of PHACE.